Epidemiología descriptiva y cambios en la frecuencia de tumores astrocíticos en el Instituto Nacional de Neurología y Neurocirugía de México / Descriptive epidemiology and frequency variations of astrocytic tumors in the National Institute of Neurology and Neurosurgery of Mexico

Resumen: Objetivo: Determinar distribución, localización y cambios de la frecuencia de tumores astrocíticos (TA) en un instituto mexicano de neurología. Material y métodos: Se revisaron los registros institucionales de TA de cinco décadas. Se compararon las relaciones TA/egresos quirúrgicos (EQ) y TA/total de tumores del sistema nervioso central (TSNC) de 1995 a 2014. Resultados: Se analizaron 2 287 TA (1 356 en hombres y 931 en mujeres). El glioma más común fue el glioblastoma multiforme (GBM), que estuvo presente en adultos jóvenes con una frecuencia mayor a la reportada en otros estudios. La relación TA/EQ y TA/TNSC fue similar entre 1995 y 2014. Conclusiones: En general, la frecuencia de TA atendidos en el Instituto es similar a la reportada internacionalmente. No obstante, los casos de TA en el subgrupo de adultos jóvenes con GBM son más frecuentes (40%) que las incidencias reportadas en otros estudios (menores al 5%). No se encontró variación significativa en la frecuencia de TA durante las últimas dos décadas.

Abstract: Objective: To determine distribution, localization and frequency variations of astrocytic tumors (AT) in a Mexican Institute of neurology. Materials and methods: Institutional registries of AT from five decades were analyzed. AT/Surgical discharges (SD) and AT/Central Nervous System Tumors (CNST) from 1995 to 2014 were compared. Results: Two thousand two hundred and eighty-seven AT (1 356 men and 931 women) were analyzed. The most common glioma was glioblastoma multiforme (GBM), found in young adults with a higher frequency to that reported in other studies. Relation of AT/SD, as well as, relation of AT/CNST was similar between 1995 and 2014. Conclusions: In general, the frequency of AT attended at the Institute is similar to that found worldwide, being only higher the number of GBM in younger adults. There was not significant variation in the frequency of AT during the time studied.

Nimotuzumab y radioterapia en el tratamiento de tumores del tallo cerebral en niños y adolescentes / Nimotuzumab and radiotherapy in the treatment of brainstem tumors in children and adolescents

Introducción: Los tumores localizados en el tallo cerebral en los niños y adolescentes conllevan un mal pronóstico, especialmente aquellos infiltrantes y difusos. Con el tratamiento de radioterapia apenas llegan a más de 15 por ciento de supervivencia y no mejora la cifra con quimioterapia agregada. Objetivos: Estimar el efecto de la asociación del tratamiento radiante con el anticuerpo monoclonal Nimotuzumab en la supervivencia de niños y adolescentes con tumores del tallo cerebral. Método: Estudio clínico no aleatorizado, analítico, longitudinal y prospectivo. Se estudió una serie de 46 pacientes entre 2 y 18 años de edad que padecían de tumores del tallo cerebral, infiltrantes y difusos, desde enero de 2008 y en seguimiento hasta marzo de 2018. Todos se trataron con radioterapia, con dosis entre 54 y 59,8 cGrey, dosis diaria de 1,8 cGrey, y se irradiaban de lunes a viernes. Mientras duró el tratamiento radiante recibieron Nimotuzumab, en la dosis de 150 mg/m2 de superficie corporal, luego semanal con 8 dosis, y finalmente mensual durante uno o dos años. Resultados: Se alcanzó en la serie una supervivencia media de 18,4 meses, y una esperada de 42,9 por ciento a 2 años y 35,5 por ciento a 5 años, estabilizada hasta los 10 años. Conclusiones: La combinación de radioterapia y el anticuerpo monoclonal Nimotuzumab incrementa la supervivencia en niños y adolescentes con tumores del tallo cerebral y es bien tolerada, aun en periodos prolongados, e incluso en casos de recidiva(AU)

Introduction: Tumors localized in the brainstem of children and adolescents entail a bad prognosis, especially those that are intrinsic and diffuse. With radiotherapy treatment, patients barely get a 15 percent of survival, and the numbers don't improve with added chemotherapy. Objectives: To estimate the effect of the association of radiotherapy treatment with Nimotuzumab monoclonal antibody in the survival of children and adolescents with brainstem tumors. . Method: Non randomized, analytical, longitudinal and prospective clinical study that was authorized by the National Regulatory Authority. There was studied a group of 46 patients aged from 2 to 18 years that suffered from intrinsic and diffuse brainstem tumors, from January 2008 (and in follow up) to March 2018. All the patients were treated with radiotherapy, with doses among 54 and 59,8 Grey, daily doses of 1,8 Grey, and from Monday to Friday. While they were under radiotherapy treatment, they get Nimotuzumab, in doses of 150 mg/m2 of corporal surface; then weekly doses of 8 shots; and finally, monthly doses during one or two years. Results: In this group there was a survival mean of 18, 4 months, and an expected survival of 42, 9 percent for 2 years and 35, 5 percent for 5 years that can be stabilized to 10 years. Conclusions: Combination of radiotherapy and Nimotuzumab monoclal antibody can increase the survival from brainstem tumors in children and adolescents(AU)

Pathological features of 78 metastatic or multicentric neoplasms involving the central nervous system in dogs / Aspectos patológicos e distribuição de 78 neoplasias metastáticas ou multicêntricas envolvendo o sistema nervoso central de caninos

Secondary neoplasms affecting the central nervous system (CNS) may occur through blood flow or direct extension. This condition occurrence has increased both due to a longer life expectancy of dogs, as well as with the employment of chemotherapeutics, which may increase the survival period and, thus, the odds of the occurrence of metastasis. The aim of this study was to analyze the epidemiological features, such as the age, breed and sex of the animals affected, as well as the gross and microscopic findings of the metastasis from neoplasms involving the CNS of 78 dogs, and, based on the data, estimate the occurrence and frequency of the metastatic or multicentric neoplasms. Females (71.79%) were most affected than males, with an average age of 9.5-years-old and a median of 10-years-old. Most of the cases had a multifocal distribution (73.07%), and epithelial neoplasms (50%) were the most common. Telencephalon was the most affected neuroanatomical region (61.1%). Mammary neoplasms were the most frequent (47.44%), followed by hemangiosarcoma (19.23%), lymphoma (10.26%) and melanoma (6.41%). Less common neoplasms included osteosarcoma, histiocytic sarcoma and lung carcinoma (2.56%). Mammary neoplasms were mainly composed of anaplastic and micropapillary carcinomas, of which only multifocal microscopic lesions were observed.(AU)

Neoplasias secundárias envolvendo o sistema nervoso central (SNC) podem ocorrer por via hematógena ou por extensão direta. A ocorrência desta condição vem aumentando devido à expectativa de vida mais longa dos cães, bem como através do uso de quimioterápicos, os quais podem ampliar a sobrevida e, portanto, as chances para a ocorrência de metástases. O objetivo deste estudo foi analisar os aspectos epidemiológicos, como idade, raça e sexo dos animais afetados, bem como caracterizar os achados macroscópicos e microscópicos de neoplasmas com metástases envolvendo o SNC de 78 caninos e, baseado nestes dados, estimar a ocorrência e a frequência de neoplasias metastáticas ou multicêntricas. Fêmeas (71,79%) foram mais afetadas do que machos, com uma idade média de 9,5 anos e mediana de 10 anos. A maior parte dos casos apresentava distribuição multifocal (73,07%), e neoplasias de origem epitelial (50%) foram as mais frequentes. A localização neuroanatômica mais afetada foi o telencéfalo (61,1%). As neoplasias mamárias foram as mais frequentes (47,44%), seguidas por hemangiossarcoma (19,23%), linfoma (10,26%) e melanoma (6,41%). Osteossarcoma, sarcoma histiocítico e carcinoma pulmonar (2,56%) foram neoplasias menos frequentes. Os tipos de neoplasmas mamários mais observados foram o carcinoma anaplásico e o carcinoma micropapilar, nesses dois tipos as lesões eram multifocais e observadas somente na microscopia.(AU)

Infecciones en pacientes con tumores sólidos de sistema nervioso central y de cabeza y cuello / Infections in patients with solid tumors of the central nervous system and head and neck

Los tumores sólidos representan el 90% de las patologías oncohematológicas que se diagnostican en Argentina. Las infecciones son una de las complicaciones más frecuentes, causando una importante morbimortalidad, y en muchos casos retrasan la prosecución de los tratamientos específicos. La incidencia y tipo de infección depende del sitio específico del tumor, los fenómenos post obstructivos, el tipo e intensidad del tratamiento que se administre, las comorbilidades del paciente y la epidemiología local, entre otros factores. En forma constante se van incorporando nuevos tratamientos al arsenal terapéutico, tales como nuevos esquemas de quimioterapia, terapias blanco e inmunoterapia, y el manejo de las complicaciones asociadas a los mismos representa un desafío para el equipo tratante.En esta revisión abordamos la epidemiología, prevención y manejo de las complicaciones infecciosas más frecuentes en los pacientes con tumores de sistema nervioso central y de cabeza y cuello

Solid tumours represent 90 percent of the oncohematologic pathologies diagnosed in Argentina. Infections are one of the most frequent complications causing important morbidity and mortality and delay in prosecution of their specific treatment. The type of infection depends on the specific site of the tumour, the presence of post obstructive phenomena, the treatment administered, comorbidity and local epidemiology, among others. New therapies are being continuously incorporated to the armamentarium of cancer treatment such as new chemotherapies regimes, target therapy and immunotherapy. The management of adverse events and infectious complications associated with them are a challenge for the physician in charge of these patients.The epidemiology, prevention and management of the most frequent infectious complications in patients with tumours of the central nervous system and head and neck are reviewed in this paper

Historical distribution of central nervous system tumors in the Mexican National Institute of Neurology and Neurosurgery / Distribución histórica de neoplasias del sistema nervioso central en el Instituto Nacional de Neurología y Neurocirugía de México

Abstract Objective: To determine the frequency of central nervous system (CNS) tumors in the first fifty years of the National Institute of Neurology and Neurosurgery of Mexico Manuel Velasco Suárez (Instituto Nacional de Neurología y Neurocirugía de México, INNN) from 1965 to 2014. Materials and methods: A total of 16 116 institutional records of CNS tumors were analyzed. The frequency and distribution of CNS tumors were evaluated by tumor type, patient age and patient gender. The annual relationship between CNS tumors and surgical discharges (SD) over the last 20 years was estimated. Results: The frequencies of most CNS tumors were consistent with those found worldwide, and the most common tumors were neuroepithelial tumors (33%), particularly astrocytic tumors (67%); meningeal tumors (26%); and pituitary tumors (20%). The incidence of pituitary tumors in these data was twice as high as that reported in other regions of the world, and the relationship between CNS tumors and SD was consistent over time (0.22-0.39). Conclusion: This study summarizes the largest sample of CNS tumor cases analyzed in Mexico and provides an important reference of the frequency of this tumor type in the country. This work will serve as a basis for conducting studies evaluating factors associated with the presence of CNS tumors and for identifying adequate public health interventions.

Resumen Objetivo: Determinar la frecuencia de neoplasias del sistema nervioso central (NSNC) en los primeros 50 años del Instituto Nacional de Neurología y Neurocirugía de México (INNN). Material y métodos: Se analizaron 16 116 registros institucionales de las NSNC, atendidas en el INNN de 1965 a 2014; se estimó su frecuencia y distribución por tipo de neoplasia, edad y género, y se determinó la relación anual de NSNC y egresos quirúrgicos (EQ) en un período de 20 años. Resultados: Las frecuencias de la mayoría de NSNC fueron consistentes con las encontradas a nivel mundial. Las más frecuentes fueron las neuroepiteliales (33%), entre las cuales destacaron las astrocíticas (67%); meníngeas (26%), e hipofisiarias (20%). El número de neoplasias hipofisiarias en esta serie fue dos veces mayor al reportado en otras regiones del mundo y la relación NSNC/EQ fue similar a través del tiempo (0.22-0.39). Conclusión: Ésta es la mayor serie de casos de NSNC analizados en México y proporciona un referente importante sobre la frecuencia de este tipo de neoplasias en el país. Este trabajo servirá de base para llevar a cabo estudios de los factores asociados a la presencia de NSNC e identificar intervenciones de salud pública adecuadas.

Epidemiologia de uma série de tumores primários do sistema nervoso central / Epidemiology of a series of primary central nervous system tumors

Objetivo: Realizar um levantamento de dados descritivos em uma série de tumores primários do sistema nervoso central. Métodos: Foram avaliados 106 casos de tumores primários do sistema nervoso central, descrevendo a idade média, os sintomas apresentados, os tipos de tumores mais frequentes e sua localização. Resultados: A idade média foi de 47 anos e 71 casos (67%) foram do sexo feminino. Observaram-se 39 tipos de tumores primários do sistema nervoso central, sendo os mais frequentes os meningiomas (51 casos, 48%), seguidos por glioblastomas (19 casos, 18%). Quanto à localização, 51 casos (48%) apresentaram-se nas meninges. Cefaleia ocorreu em 52% dos casos. Conclusões: A faixa etária da amostra é similar a de outros estudos. Observou-se predominância de meningiomas em associação à predominância do sexo feminino, no qual este tumor prevalece. Os sintomas apresentados corroboram com os descritos na literatura.

Objective: To conduct a survey of data descriptive in a series of primary central nervous system tumors. Methods: One hundred and six cases of primary central nervous system tumors were evaluated, describing the mean age, associated symptoms, the most frequent subtype of tumors and their location. Results: The mean age was 47 years and 71 cases (67%) were female. Thirty nine subtypes of primary central nervous system tumors were observed, and the most frequent subtype was meningioma (51 cases, 48%), followed by glioblastoma (19 cases, 18%). Regarding location, 51 cases (48%) were in the meninges. Headache occurred in 52% of cases. Conclusions: The mean age of the sample is similar to other studies. Meningiomas prevailed in association with female predominance, where this tumor is prevalent. The symptoms corroborate those described in the literature.

Epilepsia asociada a tumores del sistema nervioso central en niños / Epilepsy associated with central nervous system tumors in children

Introducción: La prevalencia de epilepsia en pacientes con tumores del sistema nervioso central (SNC), producto del tumor per se o secundaria al tratamiento, es mayor que en la población general. El objetivo de este estudio es analizar la frecuencia y características de la epilepsia en pacientes pediátricos con tumores del SNC. Método: Estudio descriptivo retrospectivo, realizado a través de la revisión de fichas médicas de los pacientes pediátricos con tumores de SNC entre los años 2001- 2010 en Hospital Carlos Van Buren de Valparaíso. Resultados: Revisados 97 casos pediátricos de tumores del SNC, dieciocho (18,5 por ciento eran portadores de epilepsia, 2/3 sexo masculino, promedio de edad al diagnóstico del tumor fue 7 años y de primera crisis epiléptica 6 años 7 meses. Un 61 por ciento debutó con crisis epilépticas previo al diagnóstico de tumor. Dieciseis de 18 tumores (88 por ciento) fueron supratentoriales, comprometiendo principalmente el lóbulo temporal (9 de 16). Un 83 por ciento fueron neuroepiteliales, los más frecuentes fueron astrocitomas (50 por ciento). Dieciseis casos (88 por ciento) fueron sometidos a intervención quirúrgica. En relación a la epilepsia, 73 por ciento presentó crisis parciales complejas y 38 por ciento (6 casos de 16) evolucionó con epilepsia refractaria durante el seguimiento. Hubo 3 casos que fallecieron. Conclusión: Las crisis epilépticas, sobre todo las crisis focales fueron una manifestación frecuente en este grupo de pacientes pediátricos con tumores del SNC, especialmente en aquellos de localización supratentorial, ya sea como manifestación inicial y clave para el diagnóstico de tumor o durante su evolución. Un 38 por ciento evolucionó como epilepsia refractaria. El número de intervenciones quirúrgicas y la localización tumoral incidieron en la evolución de la epilepsia. Se enfatiza la importancia de una evaluación acuciosa y búsqueda etiológica, en niños que debutan con crisis epilépticas.

Introduction: Patients with brain tumors, show a higher prevalence of epilepsy than the general population, because of the tumor itself or as a consequence of treatment. The aim of this study is to analyze the incidence and characteristics of epilepsy in patients with brain tumors. Method: Retrospective descriptive study, medical records of pediatric patients with brain tumors between the years 2001-2010 from Hospital Carlos Van Buren were reviewed. Results: From 97 patients with brain tumors, 18 (18.5 percent) presented with epilepsy. Two thirds were males. Mean age for brain tumor diagnosis was 7 years, and for first epileptic seizure 6 years 7 months. In 61 percent epileptic seizures started previous to the tumor diagnosis. Sixteen out of 18 patients (88 percent) had supratentorial, mainly temporal tumors (9/16). 83 percent were neuroepithelial, from which astrocytomas were the most frequent (50 percent). Sixteen patients had surgical treatment (88 percent). Epileptic seizures were complex partial in 73 percent. 38 percent evolved to refractory epilepsy in an average of 5 year follow-up. Discussion: Epileptic seizures, mainly complex partial seizures, were a frequent manifestation of patients with brain tumors, specially supratentorial, as the initial event or in follow up. Thirty eight per cent evolved to refractory epilepsy. Number of surgical interventions and localization of the tumor affected the evolution of epileptic seizures. The relevance of searching etiology in children who have a first epileptic seizure is emphazised.

Primary central nervous system lymphoma: A study of clinicopathological features and trend in western India.

Introduction: Primary central nervous system lymphoma (PCNSL) is rare and accounts for 1-2% of all primary intracranial tumors (ICT). There are conflicting reports regarding the increased incidence of PCNSL over the last two decades in both immunocompromised and immunocompetent patients. Aim: This study was designed to study the clinicopathological characteristics of PCNSL and to access the trend of PCNSL at our institute. Materials and Methods: All the histopathologically proven cases of PCNSL were reviewed from January 1997 to December 2009 (13 years). Immunophenotyping was performed on available paraffin-embedded tissue blocks. Immune status was evaluated and human immunodeficiency virus (HIV) serology was performed in all cases. Cerebrospinal fluid (CSF) findings were recorded whenever available. Possibility of secondary involvement by a systemic lymphoma was excluded in every case. Statistical analysis was done using χ2 -test. Results: During the study period (13 years), a total of 4715 cases of ICT were diagnosed, out of which 66 cases were PCNSL, which accounted for 1.4%. The age ranged from 10 to 75 years with a median age of 46 years. All the patients were immunocompetent. Frontal lobe was the most common site of involvement. Diffuse large B-cell lymphoma was the histological pattern in all the cases. CSF involvement was seen in only one case. Conclusions: In this study, no significant increase in the incidence of PCNSL was found at our institute. Association of PCNSL cases with HIV or acquired immunodeficiency syndrome was not found in our study.

Tumores primarios del sistema nervioso central en Cartagena, 2001- 2006 / Primary central nervous system tumours reported in Cartagena, 2001-2006

Objetivos Determinar la frecuencia de los tumores primarios del sistema nervioso central, diagnosticados en Cartagena de Indias durante el periodo de 2001-2006 y, determinar las características demográficas, epidemiológicas y clínicas de los pacientes con tumores del sistema nervioso central de una institución de la ciudad, en el mismo periodo. Métodos Se realizó un estudio descriptivo de vigilancia epidemiológica pasiva. Se tomaron los reportes de patología con diagnóstico nuevo de tumor primario del sistema nervioso central de todos laboratorios de Cartagena y se analizaron las historias clínicas disponibles de estos casos. Se estimaron las tasas de incidencia general, por año, género, grupos de edad y tipo histológico con intervalos de confianza al 95 por ciento. Además se calcularon razones estandarizadas de morbilidad. Resultados Durante los años 2001 y 2006 se encontraron 390 casos. La tasa incidencia general fue de 6,91/100 000 personas-año. El tipo histológico más frecuente fue meningioma (3,46/100 000 personas-año). Sólo se determinó la procedencia en el 43,1 por ciento de los casos. Las razones estandarizadas de morbilidad fueron más altas en Cartagena con respecto a las de los Estados Unidos, el Instituto Nacional de Cancerología de Colombia y el Registro Poblacional de Cáncer de Cali. Conclusiones Hubo una frecuencia de tumores primarios del sistema nervioso central más elevada en Cartagena que en el resto del país. Se recomienda mejorar los sistemas de registro y vigilancia para determinar la magnitud real del problema y fomentar investigaciones en busca de factores de riesgo.

Objectives Determining the frequency of primary central nervous system tumours diagnosed in Cartagena; Colombia, from 2001-2006 and determining the demographic, epidemiological and clinical characteristics of patients having central nervous system tumours reported by a single institution in Cartagena between 2001 and 2006. Methods A passive epidemiological surveillance descriptive study was carried out. The pathology reports of new diagnosed central nervous system primary tumours from all laboratories in Cartagena were taken and the available clinical records regarding these cases were analysed. The overall incidence rate and incidence rates by year, gender, age and histological type were estimated, with 95 percent confidence intervals. Standardised morbidity rates were also calculated. Results There were 390 such cases during 2001-2006. The overall incidence rate was 6.91/100,000 people-year. Meningiomas were the most frequently occurring histological types (3.46/100,000 people-year). The provenance could only be determined in 43.1 percent of cases. Standardised morbidity rates were higher in Cartagena regarding those reported in the United States and by the Colombian National Cancer Institute and the Population-based Cali Cancer Registry. Conclusions There was a higher incidence of primary central nervous system tumours in Cartagena than in the rest of the country. Registry and surveillance systems should be improved and research into risk factors encouraged.

Perfil e prevalência dos tumores primários do sistema nervoso central no Grupo Hospitalar Conceição, de Porto Alegre, RS / Prevalence and histological aspects of primary central nervous system tumors at the Grupo Hospitalar Conceição, Porto Alegre, RS

Introdução: um dos grandes focos da neuropatologia cirúrgica tem sido associar a classificação dos tumores, baseada em achados histopatológicos, com dados clínicos e imuno-histoquímicos a fim de determinar entidades clínico-patológicas. Avanços nas técnicas de neuroimagem permitem, atualmente, determinar com precisão a localização da lesão e a realização de biópsias em múltiplas áreas deste grupo heterogêneo de tumores. Com o objetivo de estimar a associação entre tipo histológico e topografia, os autores descrevem todos os casos de tumores primários do sistema nervoso central avaliados no Hospital Conceição. Metodologia: foram avaliados 912 casos distintos de tumores primários do sistema nervoso central, entre 1995 e 2009, no laboratório de patologia do Grupo Hospitalar Conceição. Os autores descrevem os tipos histológicos encontrados e sua associação com topografia, idade, sexo e achados imuno-histoquímicos. Resultados: os tipos histológicos mais comuns foram o glioblastoma (31,15%), astrocitoma difuso (10,86%) e meningioma (grau I da OMS - 10,75%), com associação destes com a topografia (p=0,001) e a idade dos pacientes (p=0,01). A avaliação imuno-histoquímica foi realizada em 48 casos de neoplasia maligna indiferenciada, sendo que a expressão dos anticorpos determinou a diferenciação tumoral destas lesões, tornando este método fundamental na avaliação deste subgrupo de tumores. Conclusões: o presente estudo sugere a associação entre tipo histológico, idade e topografia em casos de tumores primários do sistema nervoso central

Introduction: One of the major targets of surgical neuropathology has been to associate tumor classification, based on histopathological findings, with clinical and immunohistochemical data in order to determine clinico-pathological entities. Current advances in the neuroimaging techniques allow to accurately determine the location of lesion and to perform biopsies at multiple sites of this heterogeneous group of tumors. In order to assess the association of this histological type with topography, the authors describe all cases of primary tumors of the central nervous system evaluated in the Conceição Hospital. Methods: 912 distinct cases of primary tumors of the central nervous system were evaluated in the laboratory of Pathology of the Grupo Hospitalar Conceição between 1995 and 2009. The authors describe the histological types found and their association with topography, age, sex and immunohistochemical findings. Results: The most common histological types were the glioblastoma (31.15%), diffuse astrocytoma (10.86%) and meningioma (grade I of WHO, 10.75%), with association of these with topography (p=0.001) and patient age (p=0.01). Immunohistochemical evaluation was performed in 48 cases of undifferentiated malignant neoplasia, where antibody expression determined tumor differentiation

Therapy for glioma: Indian perspective.

Central nervous system (CNS) are rare neoplasms with considerable heterogeneity and variation. The most common primary lesions of CNS are gliomas. A majority of the data about the demography and management of gliomas has emerged from the west. However, there may be considerable variation in the presentation, behavior, and response to treatment between patients in the western world and the Asian population. This article discusses gliomas with special reference to data from oncology centers in India.

Apresentação clínica dos tumores sólidos mais comuns na infância e adolescência / Clinical presentation of the most common solid tumors in childhood and adolescent

O câncer infanto-juvenil é uma doença relativamente rara, sendo responsável por 0,5-3% do total de casos de câncer. Entretanto, ainda é causa de grande morbimortalidade em pacientes nessa faixa etária em nosso meio. Diagnóstico precoce e tratamento em centros especializados são essenciais para aumento da sobrevida em longo prazo. Objetivo: alertar o pediatra a respeito da importância do reconhecimento precoce das principais neoplasias malignas da infância e adolescência, dando ênfance à epedemiologia e apresentação clínica dos tumores sólidos. Métodos: foram selecionados 26 referências bibliográficas em pesquisa realizada nas base de dados Medline e no LILACS, em português, espanhol e inglês, utilizando-se as palavras-chave câncer infanto-juvenil, apresentação clínica, sobrevida.

Histological spectrum of ependymomas and correlation of p53 and Ki-67 expression with ependymoma grade and subtype.

BACKGROUND: Clinical and histological criteria for ependymoma prognosis are well recognized. Recently few studies have been done based on Immunohistochemistry for prognostication of these tumours. In this study we have correlated the histological spectrum with immmunoexpression of p53 and Ki67 in these tumors. AIMS: To know the incidence of ependymomas; study their morphological spectrum and to evaluate expression of P53 and Ki 67 in different morphological subtypes. MATERIAL AND METHOD: A retrospective study was preformed on 70 ependymomas received in a period between 1994 and 2001. Entire tissue received was processed for routine paraffin embedded H&E stained sections. Immunocytochemistry was performed using antibodies to GFAP, EMA, Pancytokeratin and synaptophysin, to differentiate papillary ependymoma from choroid plexus papilloma; clear cell ependymoma from oligodendroglioma and central neurocytoma; ependymoblastoma from other embryonal tumours. p53 and Ki-67 immunohistochemistry was performed to correlate their expression with various tumour grades and subtypes. RESULTS: There were 3 cases (4.2%) of Grade I ependymoma (2 cases of myxopapillary ependymoma and 1 case of subependymoma); 57 cases (81.5%) of ependymoma grade II (43 of these were of classical variety, 11 of clear cell ependymoma, 2 of papillary and 1 case of cellular ependymoma). There were 9 cases (12.8%) of anaplastic ependymoma (one of these was a clear cell ependymoma and 1 case (1.5%) of ependymoblastoma CONCLUSION: p53 and Ki67 indices can be used in routine diagnostic laboratories to supplement the tumor grade on histology and more studies with follow up should be performed to analyse the prognosis of different subtypes. The expression of Ki 67 and p53 was significantly higher in anaplastic ependymomas. 4 out of 11 cases of clear cell ependymomas showed higher Ki 67 indices as compared to classical grade II ependymomas, thus further highlighting the importance of differentiating the various subtypes.

Histological pattern of central nervous system neoplasms

To see in a large series of patients the histological pattern of CMS neoplasms and to provide a comprehensive data about the frequency of CMS neoplasms in Pakistani patients and to give a clearer idea about their prevalence. Retrospective study of all consecutive cases of CNS neoplasms diagnosed at The Aga Khan University Hospital [AKUH] between 1[st] January, 1994 and 31st December, 1999. The section of Histopathology, AKUH, Karachi.. Our series included a large number of cases i.e., 1110. There are very few local studies on CNS tumours and none so large. Our data was correlated with published Western and Pakistani data. Glial tumours were the commonest primary CNS neoplasms followed by meningiomas. Among glial tumours, astrocytomas comprised the largest group and the majority was high grade [III and IV] astrocytomas. Percentages of anaplastic oligodendrogliomas and ependymomas were also significant. Male to female ratio was 1.6:1. However, meningiomas were more common in females. Almost half of the CNS tumours in patients 15 years or younger were in the posterior cranial fossa. Metastatic tumours were much lower as compared to the West. Except for the high percentages of anaplastic oligodendrogliomas and ependymomas and the low percentage of metastatic tumours, most of our findings roughly correspond with the published data

Oligodendrogliomas: estudo anatomopatológico e clínico de 15 casos / Oligodendroglioma: a pathological and clinical study o 15 cases

Oligodendrogliomas correspondem a 4-5 por cento dos tumores primários do sistema nervoso central apresentando crescimento infiltrativo e lento. Relatamos os achados anatomopatológicos e clínicos de 15 casos de oligodendrogliomas. Oito pacientes eram do sexo masculino e 7 do feminino. As idade oscilaram entre 17 e 66 anos, apresentando média de 39,73 anos. A sintomatologia apresentada correspondeu ao crescimento expansivo, sendo cefaléia (60 por cento) e crises convulsivas (60 por cento) os sintomas mais frequentes. O lobo frontal (n=6) foi o sítio anatômico mais acometido, seguido pelo parietal (n=2), temporal (n=1) e occipital (n=1). Cinco pacientes foram submetidos a ressecção total do tumor e 10 pacientes a exerese parcial; dentre estes, 3 foram submetidos a radioterapia adjuvante, 1 a quimioterapia e 1 a quimio e radioterapia. Evidenciou-se taxa de recidiva tumoral total de 60 por cento em período médio de 32 meses de acompanhamento. Cinco recidivas tumorais ocorreram nos pacientes submetidos apenas ao tratamento cirúrgico e quatro nos pacientes submetidos a quimio ou radioterapia adjuvante. Estes achados aproximam-se dos encontrados na literatura, auxiliando na compreensão do comportamento biológico deste raro tumor cerebral.

Ependinomas: achados clínicos, epidemiologicos e anatomapatológicos de 22 casos / Ependymonas: Clinical, epidemiological and findings of 22 cases

Ependimomas são neoplasias compostas por células ependimárias neoplásicas, manifestando-se predominantemente em crianças e adultos jovens. Relatamos os achados clínicos, epidemiológicos e anatomopatológicos de 22 casos de ependimomas. Dos 22 casos analisados, 14 ocorreram em pacientes do sexo masculino e 8 em pacientes femininos. As idades variaram de 1 a 58 anos, com média de 24,63 anos. Quanto à localização, 10 casos acometeram medula, 7 ocorreram em hemisférios cerebrais, 2 intraventriculares e 1 no tronco cerebral. Os sinais e sintomas observados incluiram redução da força muscular (59,1 por cento dos pacientes), alterações da marcha (36,3 por cento), alterações da sensibilidade (36,3 por cento), hiperreflexia e sindrome de hipertensão endocraniana, com período de evolução variando de 15 dias a 8 anos, com média de 17,58 meses. Sete pacientes foram submetidos a ressecção cirúrgica total, 7 a exérese parcial, 4 a exérese parcial associada a radioterapia adjuvante, 3 a ressecção parcial seguida de quimioterapia e 1 a tumorectomia total associada a radioterapia. Evidenciou-se taxa de recidiva tumoral total de 18,2 por cento. Estes achados aproximam-se dos encontrados na literatura internacional, corroborando para a compreensão do comportamento biológico deste tumor.

Meningiomas: estudo epidemiológico e anátomo-patológico de 304 casos / Meningiomas: epidemiological and anatomopathological study of 304 cases

No presente estudo, foram pesquisados os tumores do sistema nervoso central (SNC) diagnosticados na cidade de Curitiba entre 1990 e 1994. Realizaram-se 2371 biópsias de SNC das quais 1340 casos eram neoplasias primárias. Dentre as neoplasias primárias de SNC 304 (22,6 por cento) eram meningiomas. Duzentos e nove meningiomas ocorreram em mulheres (68,7 por cento) e 95 ocorreram em homens (31,3 por cento). As idades variaram de 3 a 90 anos, com média de 45,8 anos. Duzentos e oitenta meningiomas localizavam-se no cérebro, 10 na medula espinhal, 9 no cerebelo e 5 na emergência de nervos cranianos. Na classificaçao histológica verificou-se que 294 (96,7 por cento) eram do tipo clássico, 6 malignos (ou anaplásicos), 3 atípicos e 1 papilar. No grupo dos meningiomas clássicos 267 eram meningoteliais, 10 psamomatosos, 5 fibroblásticos, 5 microcísticos, 5 transicionais e 2 angiomatosos. Os autores concluem que as neoplasias meníngeas representaram o grupo mais frequente de tumores primários do SNC e apresentaram características epidemiológicas e anátomo-patológicas similares às da literatura mundial, na qual é dada ênfase à dificuldade de estabelecer critérios biológicos e histológicos de malignidade neste grupo de tumores.

Epidemiología de las neoplasias malignas en niños residentes del Distrito Federal (1982-1991) / Epidemiology of malignant neoplasia in children from Mexico City (1982-1991)

Introducción. Poco se conoce en México sobre la epidemiología de las neoplasias malignas en niños. El objetivo de este estudio fue determinar la incidencia, tendencia y otras características epidemiológicas de las neoplasias malignas en niños residentes del Distrito Federal (DF) durante el período 1982-1991. Material y métodos. Tipo de estudio: encuesta hospitalaria. población de estudio: expedientes de los 6 principales hospitales que atienden niños con neoplasias malignas en el DF. Resultados. Se estudió un total de 1069 casos. Hubo una tendencia al incremento de las neoplasias (tasa en 1982 de 24.08 y en 1991 de 69.8 x 10 6); asimismo hubo tendencia para las leucemias, enfermedad de Hodgkin, histiocitosis, tumores del sistema nervioso central (TSNC), óseos y de células germinales (TCG). La relación hombre/mujer fue de 1.4. En 1991 la incidencia más alta fue para los niños menores de 5 años; para este año la incidencia más alta fue para las leucemias (23.3), linfomas (10.8), retinoblastoma (6.2), TSNC y TCG (5.8). La incidencia más alta se encontró en las delegaciones Benito Juárez e Iztacalco; la edad más frecuente de los padres fue entre 20 y 30 años; las ocupaciones más frecuentes fueron obreros y profesionista; el antecedente familiar de cáncer fue del 16.9 por ciento. El 96.8 por ciento de los casos nació de embarazo de términó y sólo el 5.8 por ciento nació con bajo peso. Conclusiones. Se encontró una incidencia menor a la reportada mundialmente, pero una tendencia al incremento. La mayor incidencia se encontró en los niños menores de 5 años: Asimismo, se encontró consistencia con el patrón latinoamericano de neoplasias y otros datos consistentes con lo reportado en la literatura internacional

Tumores primários do sistema nervoso central: aspectos epidemiológicos / Primary tumors of central nervous system: epidemiological aspects

Os coeficientes de incidência dos tumores primários do sistema nervoso advindos de estudos com possibilidade de captaçåo mais amplas dos casos våo de 5,0 a 15,7 por 100.000 habitantes. Eles tendem a aumentar com a idade (com exceçåo do meduloblastoma e ependimoma que acometem crianças ou pessoas mais jovens, respectivamente) o que é especialmente considerável com os meningiomas (mais em mulheres), gliomas (mais em homens), neurilenoma e adenomas cromófobos. De modo geral o predomínio desses tumores é no homem (com nítida exceçåo dos meningiomas), mais do encéfalo do que da medula, mais primários do que metastáticos. Usualmente é referida a primazia dos gliomas, sobre os meningiomas, eventualmentepela maior facilidade diagnóstica dos primeiros. Os coeficientes específicos por idade costumam chegar a um máximo nos 50-69 anos com exceçåo dos achados de Rochester e Islandia que tendem a demonstrar maiores índices nos muitos idosos. Vários estudos observam tendências temporais crescentes nos tumores cerebrais especialmente nos idosos. Talvez isto ocorra devido a maior disponibilidade de recursos diagnósticos e mudança mais recentemente na cobertura de saúde refletindo em parte novos valores sociais (especialmente em relaçåo aos idosos). Såo considerados dados nacionais advindos de registros de mortalidade e de tumores

Tumores supratentoriales infantiles / Tumors supratentorials in children

Se revisan los tumores del sistema nervioso central (S.N.C) en el lapso comprendido entre 19644-1992 del Hospital de niños de Caracas. La estadística demuestra que en 17 años (1964-80), época pre-tomográfica se trataron quirúrgicaamente 159 casos (9,35 casos anuales). En 12 años (1981-92) se atendieron 341 casos el doble en menos tiempo (28,40 casos por año) gracias a la tomografía. El total de 29 años fue de 510 casos o sea 17,8 casos por año, cifra inferior a la demanda. Supratentorial: 194 casos (38,09 por ciento) e infratentoriales 283 (55,6 por ciento); espinales: 33 (6,47 por ciento). El aspecto tentorial supratentorial fue lo siguiente: Astrocitomas: 52 casos (10 por ciento), craniofaringioma 22 (4,10 por ciento), Ependinomas: 16 (3,13 por ciento), Pinealomas: 12 (2,34 por ciento), Nervio y Quiasma óptico: 12 (2,34 por ciento), Neuroectodérmico primitivo: 10 (1,96 por ciento), Plexo Coroide: 9 (1,76 por ciento), Teratoma: 6 (1,17 por ciento), Glioblastoma multiforme: 4 (0,78 por ciento), Meningioma: 4 (0,78 por ciento), Oligodendroglioma: 3 (0,58 por ciento), Hemangioma: 3 (0,58 por ciento), Sarcoma: 3 (0,58 por ciento), Esponglioblastoma: 1 (0,19 por ciento). Localización: Hemisféricos: 82 (16,04 por ciento), Ventriculares 14 2,74 por ciento. Los postulados oncológicos rigen la conducta quirúrgica de estas lesiones: a) Eliminación de los síntomas, b) Preservación anatómica, c)Reducción al máximo o eliminación de población tumoral, d) Eliminación de tejidos necroticos con células. Toda lesión, no importa su ubicación, debe tener biopsia donde la estereotaxia encuentra su aplicación en lesiones profundas y de tallo cerebral